PubMed · 14074737
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Z KAMINSKI, L DYDYK, L PAWLICKA. 1963. [MEDIASTINAL CHEMODECTOMA].. https://pubmed.ncbi.nlm.nih.gov/14074737/
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The most common sites of paragangliomas occurring in the head and neck region are the carotid body and vagal glomus. The clinical course of carotid body paragangliomas is characterized by two modes of development: locoregional growth and distant metastatic dissemination. Few cases of malignant carotid body paraganglioma have been reported in the literature. We report a case managed in the ENT Department of Grenoble university hospital in 1993. A 53-year-old woman presented a bilateral functional (dopamine secretion) carotid tumor, stage II on the right side and stage I on the left side according to the Shamblin classification. Two successive excisions with complete resection were performed without complication. Two years after the second surgical excision, bone and liver metastases were diagnosed and visualized with a Ostreoscan scintigraphy. Clinical course was rapidly unfavorable and the patient died a few weeks later. Malignant forms of carotid paraganglioma are very uncommon and there is no cellular or histological appearance significantly contributing to the diagnosis of malignancy. Clinical findings are the most reliable criteria for malignancy, with the development of distant metastases usually after about 9 years. Long follow-up is essential. Histology in needed to establish the diagnosis of metastasis. A histology specimen may not be possible for a functional paraganglioma. In this case scintigraphy can provide the diagnosis. Malignant forms are more frequent in case of functional paraganglioma, more particularly in case of dopamine secretion.
The carotid body (CB) is a highly specialized small organ located at the bifurcation of the common carotid artery in the neck and plays an important role in acute adaptation to hypoxia. The most common diseased state of the carotid body is its enlargement (i.e., the CB paraganglioma), which can be caused by a genetic predisposition (hereditary paraganglioma, PGL) and by chronic hypoxic stimulation. The CB is the most common tumor site in head and neck paragangliomas. Currently, inactivating germline mutations in the mitochondrial complex II subunits SDHB, SDHC, and SDHD have been identified as genetic risk factors for CB tumors (CBTs). Another locus at chromosome 11q13, identified by linkage analysis in a single family, may harbor a fourth susceptibility gene. Although CBTs are mostly slow-growing and benign, they can cause significant morbidity because of their proximity to major arteries and nerves in the head and neck. Here, we review the etiological factors implicated in the development of CBTs and provide information pertaining to their clinical presentation. Although CBTs are rare, they have the potential to provide unique insights for tumorigenesis and oxygen sensing and signaling mechanisms.
Our objective was to evaluate tumor hemodynamics of paragangliomas of the head and neck. A 2D magnetic resonance projection angiography (MRPA) protocol was developed. Six patients with histologically proven paragangliomas were included in the study. The sequence protocol and data post-processing procedure were optimized in view of a high temporal resolution and maximum contrast-to-noise ratio. Image-time series of two freely orientated slabs could be obtained. Correlation analysis was performed to generate selective arterial and venous weighted angiographic images. Glomus tumors showed a rapid and intense homogeneous enhancement following the intravenous administration of contrast material. The smallest tumors investigated measured 10 mm and could be visualized clearly. Time-resolved analysis of the tumor enhancement revealed an early bolus arrival within the vascular bed of the tumor and prolonged incomplete washout indicating blood pooling. The dynamic contrast enhancement of glomus tumors can be evaluated non-invasively with MRPA providing a high temporal resolution and high image quality. The characteristic contrast enhancement of glomus tumors can be helpful in the diagnostic workup of lesions that may mimic glomus tumors.